Author(s): Alexandre, André ; Furtado, Inês ; Gonçalves, Fabienne ; Gonçalves, Fabienne ; Melo, Alzira ; Alves, Joana ; Santos, Mario ; Reis, Abilio
Date: 2023
Persistent ID: http://hdl.handle.net/10400.16/2990
Origin: Repositório Científico da Unidade Local de Saúde de Santo António (ULSSA)
Subject(s): Idiopathic pulmonary arterial hypertension; Intravenous epoprostenol; Oral selexipag; Pulmonary hypertension; Switchback therapy; Transition therapy
Intravenous (i.v.) prostacyclin is the cornerstone treatment in high-risk pulmonary arterial hypertension (PAH) patients. Selexipag is an orally available prostacyclin receptor agonist. Limited data are available regarding the feasibility of transitioning from i.v. epoprostenol to selexipag. A 50-year-old woman with idiopathic PAH was diagnosed in a World Health Organization (WHO) Functional Class (FC) IV. She improved with upfront triple combination therapy, including i.v. epoprostenol. Over 2 years of follow-up, the patient remained at low risk and expressed strong preference towards oral therapies. After careful risk-benefit clinical consideration, she was transitioned from i.v. epoprostenol to selexipag. Selexipag was started at dosage of 200 μg twice daily (b.i.d.) and titrated up to 1600 μg b.i.d. over 8 weeks (up-titration of 200 μg b.i.d. every week). Simultaneously, i.v. epoprostenol was down-titrated 3.0 ng/kg/min every week from a dosage of 27.5 ng/kg/min. The transition occurred under strict medical surveillance and was well tolerated. One year after discontinuation of epoprostenol, the patient remains in WHO FC I and has no signs of clinical deterioration. Although not generalizable to most PAH patients, this case highlights that a carefully planned transition from epoprostenol to selexipag is feasible in selected low-risk patients within a shared medical decision-making framework.
